Words similar to fibrillin
Example sentences for: fibrillin
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Each has a signal sequence (55 amino acids and 49, respectively), a second domain containing an EGF-like repeat, a proline/glycine rich region, a fibrillin motif [ 17 ] , an EGF calcium-binding repeat, and a TGF-β repeat (390 and 389 amino acids, respectively), a third domain containing a proline/glycine rich region (113 and 111 amino acids, respectively), a fourth domain containing numerous calcium-binding type II EGF-like modules (D/N)(I/V)(D/N)(E/D)C 1 (678 and 677 amino acids, respectively) and a fifth carboxyl-terminal domain (5 and 21 amino acids, respectively) (human LTBP-3 information was obtained from the GenBank database accession no.
Primary fibroblast cultures from patients and control individuals were grown to confluency and then metabolically labeled with 35S-cysteine as described [ 17 18 ] . The soluble cell lysis fraction, containing newly synthesized labeled fibrillin-1 molecules, and the insoluble fraction, representing the labeled fibrillin in the extracellular matrix, were fractionated by SDS polyacrylamide gel electrophoresis and autoradiographed.
The deposition of newly synthesized fibrillin into the matrix was greatly reduced (13 % of normal), however, suggesting that the internally deleted mutant fibrillin-1 molecules are secreted and exert a dominant-negative effect on extracellular microfibril assembly.
In Case 2 with del exons 44-46, fibrillin synthesis was normal (109% of control), indicating that the mutant fibrillin molecules are stable.
This hypothesis is not only based on analysis of Case 1 reported here but on a similar analysis of five previously reported cases, one with exon 51 skipping [ 19 ] and four with cysteine substitutions immediately N-terminal of the LTBP like domains [ 9 ] . It is also possible, however, that proteolytic fragments of the mutant protein associate with the normal length fibrillin molecules and inhibit their secretion.
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